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PubMed1997

Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas.

OBJECTIVE: In 1940 Kasabach and Merritt described an infant with a vascular anomaly, extensive purpura, and thrombocytopenia; they called his lesion "capillary hemangioma." Hemangioma is a benign tumor that grows in infancy and is characterized by proliferation of endothelial cells and regression during childhood. Although Kasabach-Merritt syndrome (KMS) is frequently mentioned as a possible complication of hemangioma, our experience suggests that the anatomic vascular lesion underlying the thrombocytopenia is not a "true," classic, involuting type of hemangioma of infancy and childhood. STUDY DESIGN: We reviewed the clinical and hemostasis data and the response to treatment in 22 cases of KMS, and we analyzed the biopsy specimens of 15 of them. RESULTS: Clinically none of the 22 patients had classic hemangioma. There was no female preponderance. All patients had severe thrombocytopenia (lowest platelet count = 3000/mm3) and consumption of fibrinogen. Histologically, none had the typical "capillary," involuting type of hemangioma of infancy: they exhibited either a tufted angioma or a kaposiform hemangioendothelioma pattern; all specimens also contained numerous abnormal lymphatic-like vessels; lymphatic malformation was the major component in two patients. The infants exhibited a heterogeneous response to a number of therapeutic regimens, as noted in other reports. Severe morbidity was present; three of our patients died, and one had leg amputation. "Residua" were, in fact, residual vascular neoplasia, variable in duration, and not a stable fibrofatty residuum, as in classic involuted hemangioma; only the hematologic phenomenon was "cured" after a period of years. CONCLUSIONS: KMS is a distinctive disease of infancy, but the underlying vascular lesion is not a "true," classic, involuting type of hemangioma of infancy. This is a different vascular tumor with a resemblance pathologically to either tufted angioma or kaposiform hemangioendothelioma in association with lymphatic-like vessels. Whether the underlying lesion in KMS is a single anatomic entity or heterogeneous cannot be definitely concluded from this study. We need a better understanding of the pathogenesis of KMS to improve our therapeutic management.

Biopsy↗
PubMed2000

Low infectious complications in segmental living related small bowel transplantation in adults.

Clinical small bowel transplantation (SBTx) has been associated with a high rate of infectious complications. Laparotomy, preservation injury, abnormal motility, lymphatic disruption, aberrant systemic venous drainage, rejection and antibiotic therapy could all be implicated in the etiology of these complications. In addition to the underlying disease, total parental nutrition could determine infections and liver impairment. Recently, standardized techniques for segmental living related SBTx (LR-SBTx) have been developed. This technique allows reduction of some of these factors, thus resulting in a reduced incidence of infections. We report the infectious complications observed in 3 patients with short bowel syndrome treated with LR-SBTx at our institution. A segment of 180-200 cm of ileum was transplanted with a neglectably short cold ischemia time (CIT). The donor bowel was decontaminated. Oral tacrolimus, prednisone and IV induction with ATG were used for immunosuppression. Blood, stool, urine, sputum and peritoneal fluids were collected and cultured as a routine surveillance. All recipients are alive with a current follow-up time up to 21 months. No bacterial infections were observed during the post-transplant period. One patient developed cytomegalovirus (CMV) enteritis 4 months after LR-SBTx and was treated successfully with IV ganciclovir. In our limited experience, LR-SBTx is associated with a low infection rate. This could be due to optimal graft decontamination, short CIT and to the reduced incidence of rejection and lower immunosuppression used in this immunologically well-matched combination.

Adult↗
PubMed1998

Increased microvascular density and enhanced leukocyte rolling and adhesion in the skin of VEGF transgenic mice.

Vascular endothelial growth factor (VEGF) has been implicated in the pathologic angiogenesis observed in psoriasis and other chronic inflammatory skin diseases that are characterized by enhanced expression of VEGF by epidermal keratinocytes and of VEGF receptors by tortuous microvessels in the upper dermis. To investigate the functional importance of chronic VEGF overexpression in vivo, we used a keratin 14 promoter expression cassette containing the gene for murine VEGF164 to selectively target VEGF expression to basal epidermal keratinocytes in transgenic mice. These mice demonstrated an increased density of tortuous cutaneous blood capillaries with elevated expression levels of the high affinity VEGF receptors, VEGFR-1 and VEGFR-2, most prominently during the neonatal period. In contrast, no abnormalities of lymphatic vessels were detected. In addition, the number of mast cells in the upper dermis was significantly increased in transgenic skin. Intravital fluorescence microscopy revealed highly increased leukocyte rolling and adhesion in postcapillary skin venules that were both inhibited after injection of blocking antibodies against E- and P-selectin. Combined blocking antibodies against intercellular adhesion molecule-1 and lymphocyte function-associated antigen-1 were without effect, whereas an anti-vascular cell adhesion molecule-1/VLA-4 antibody combination almost completely normalized the enhanced leukocyte adhesion in transgenic mice. This study reveals VEGF as a growth factor specific for blood vessels, but not lymphatic vessels, and demonstrates that chronic orthotopic overexpression of VEGF in the epidermis is sufficient to induce cardinal features of chronic skin inflammation, providing a molecular link between angiogenesis, mast cell accumulation, and leukocyte recruitment to sites of inflammation.

Animals↗
PubMed2000

Pulmonary lymphangiectasia revisited.

PURPOSE: Pulmonary lymphangiectasia (PL) is a rare, poorly documented disease characterized by abnormal pulmonary lymphatics. Although case reports are published, little is known about survivors past the neonatal period. METHODS: This is a retrospective review of histologically proven PL in fetuses, infants, and long term survivors since 1965. RESULTS: Eleven children (8 boys, 3 girls) and 8 aborted fetuses (7 male, 1 female) were identified. The fetuses weighed 463.4 g (177 to 681 g). Six were aborted between 19 to 24 weeks of gestation for multiple malformations or anencephaly, and 2 spontaneously aborted: one with PL only, the other with twin-twin transfusion syndrome. Clinical PL was diagnosed between 0 and 11 months of age. Six children died (2 neonatal, 4 within 10 days), 5 survived. Two deaths occurred after cardiac surgery. Among survivors, the symptomatology and frequency of admissions diminished over time. Symptoms included progressive respiratory distress, chronic cough, recurrent pneumonia, bronchial asthma, and choking. One child with bilateral chylothorax was later diagnosed with Noonan syndrome; 2 patients had minor cardiac malformations. Rapid deterioration occurred with mild respiratory infections with only supportive treatment available. Chest x-ray showed marked hyperinflation with interstitial infiltrate. CONCLUSIONS: This is the first long-term study of primary PL and will help counsel parents. Although fatal in the neonatal period, survival is possible if diagnosed past the neonatal period and improvement is expected.

Abortion, Spontaneous↗
PubMed2006

Treatment of cystic lymphatic vascular malformations with OK-432 sclerotherapy.

BACKGROUND: Cystic lymphatic vascular malformations are benign lesions that can cause disfigurement and functional impairment. Complete surgical resection is often difficult, and clinical recurrence is common. Sclerotherapy has been used as an alternative to excision. OK-432 is a lyophilized mixture of Streptococcus pyogenes and benzylpenicillin which, when injected into a lesion, has shown significant ability to reduce its size or obliterate it completely. METHODS: The authors report a series of 12 patients treated in this fashion at the Vascular Anomalies Clinic, British Columbia Children's Hospital, between 1999 and 2004. All patients underwent imaging of the lesion: 10 had magnetic resonance imaging, one had a computed tomographic scan, and one had ultrasound examination. Six patients had macrocystic malformations (cysts > or = 2 cm) and six had microcystic or combined lymphaticovenous malformations. Patients were treated with intralesional injection of OK-432. The position of the injection was confirmed by angiography and/or ultrasound in 10 cases. Response to treatment was assessed clinically. RESULTS: All patients with macrocystic malformations had complete resolution or good response to treatment. None required any additional treatment. In contrast, those with microcystic or combined malformations responded poorly. All of these patients underwent subsequent excision without adverse consequences. The size and location of the lesion did not correlate with response to treatment. Seventy-five percent of patients experienced pyrexia. Local swelling is an expected phenomenon and must be anticipated, particularly for lesions near the airway. CONCLUSIONS: OK-432 is an excellent treatment for patients with macrocystic lymphatic malformations. However, it is ineffective for microcystic lesions.

Child↗
PubMed2006

Percutaneous sclerotherapy for lymphatic malformations: a retrospective analysis of patient-evaluated improvement.

PURPOSE: To evaluate the midterm outcomes of percutaneous sclerotherapy of lymphatic malformations (LMs) as judged by patients. MATERIALS AND METHODS: A 13-item survey questionnaire was sent to 74 patients who had undergone at least one sclerotherapy procedure in our hospital from January 1997 through January 2003. Information regarding the anatomic location, specific symptoms reported, history, treatment satisfaction, postprocedural complications, and number of treatment sessions was elicited. Four sclerosing agents (as single agents or in combination with other agents) were used: ethanol, sodium tetradecyl sulfate 3% (STS), OK-432, and doxycycline. RESULTS: Fifty-five patients or their caregivers completed the survey. The patients' ages ranged from 6 months to 48 years at the time of the first procedure (mean, 12 y; median, 4 y). A majority of LMs were located in the cervicofacial region. The size and location of the lesion, recurrent infection, and pain were the most frequent indications for treatment. Fifty-one percent of these patients received sclerotherapy alone or in conjunction with surgery as primary treatment. Ethanol was the most common sclerosing agent used, followed by doxycycline, STS, and OK-432. Response varied with the type of LM, with 100%, 86%, and 43% of the patients reporting good to complete response for macrocystic, microcystic, and combined-type LMs, respectively. Skin blistering and ulcers were the most common complications. Permanent complications were uncommon and were largely related to ethanol use. CONCLUSIONS: Percutaneous sclerotherapy provides effective midterm primary treatment for LMs. Treatment outcomes appear to vary according to the morphology of the malformation.

Adolescent↗
PubMed2005

Enamel hypoplasia of the primary dentition in a 4-year-old with intestinal lymphangiectasia.

Intestinal lymphangiectasia (IL) is a rare disorder, and its incidence and prevalence is unknown for either Australia or world-wide. It is characterized by diarrhoea, mild steatorrhoea, oedema, enteric loss of protein (protein-losing enteropathy) and abnormal dilated lymphatic channels in the small intestine. Whilst oedema and diarrhoea are the predominant clinical features, other observed features include hypoalbuminemia, hypogammaglobulinemia, trace metal deficiency, hypocalcemia and chylous pleural effusions. While medical presentation of the condition has been reported widely, few descriptions of oral findings have been published. A search of Medline found two reports of dental findings in the permanent dentition in patients with IL. To date, there have been no reports on dental findings in the primary dentition. The primary dentition of a 4-year-old boy with IL had teeth with enamel defects which reflected the timing of enamel development and the period in which the disease was active. The present report highlights the need for early involvement of the dental team in the dental management of children with IL.

Child, Preschool↗
PubMed2006

Histopathological reporting of paediatric cutaneous vascular anomalies in relation to proposed multidisciplinary classification system.

BACKGROUND: The terminology applied to vascular anomalies has been variable in previously published literature making interpretation suboptimal. The International Society for the Study of Vascular Anomalies (ISSVA) has proposed a revised classification based on clinical features and histopathological findings. This classification is increasingly being accepted as clinically useful and a platform for future studies. AIMS: To examine the extent to which the ISSVA classification can be practically applied to diagnostic histopathological specimens. METHODS: Cutaneous vascular lesions received in a single paediatric pathology unit during a 2-year period (2004-5) were reviewed, including glucose transporter protein 1 (GLUT1) immunostaining where required, and lesions were reclassified according to the ISSVA classification. RESULTS: 144 specimens were identified. Appropriate full clinical information was provided in only 17% of cases at submission. Infantile haemangiomas comprised 46% of cases, 18% of which were regressive type, initially inaccurately identified as vascular malformations before GLUT1 immunostaining. 30% of lymphatic malformations and all lymphovenous malformations were previously classified as vascular malformations, not otherwise specified. CONCLUSIONS: The ISSVA classification of vascular anomalies provides a useful framework for histopathologists to classify vascular anomalies. However, meaningful and appropriate use of such a system is dependent on the adequacy of clinical information provided and routine use of immunohistochemical markers.

Biomarkers, Tumor↗
PubMed2003

A case of solitary pulmonary lymphangioma.

Solitary pulmonary lymphangiomas are rare benign lesions thought to result from the development of abnormally proliferating lymphatic vessels. This report describes a case of solitary pulmonary lymphangioma resected under video assisted thoracoscopic surgery and diagnosed using histological and immunohistochemical investigations.

Female↗
PubMed2024

Central conducting lymphatic anomaly: from bench to bedside.

Central conducting lymphatic anomaly (CCLA) is a complex lymphatic anomaly characterized by abnormalities of the central lymphatics and may present with nonimmune fetal hydrops, chylothorax, chylous ascites, or lymphedema. CCLA has historically been difficult to diagnose and treat; however, recent advances in imaging, such as dynamic contrast magnetic resonance lymphangiography, and in genomics, such as deep sequencing and utilization of cell-free DNA, have improved diagnosis and refined both genotype and phenotype. Furthermore, in vitro and in vivo models have confirmed genetic causes of CCLA, defined the underlying pathogenesis, and facilitated personalized medicine to improve outcomes. Basic, translational, and clinical science are essential for a bedside-to-bench and back approach for CCLA.

Cell-Free Nucleic Acids↗
PubMed2002

Primary intestinal and thoracic lymphangiectasia: a response to antiplasmin therapy.

Lymphangiectasia is a congenital or acquired disorder characterized by abnormal, dilated lymphatics with a variable age of presentation. We describe a case of lymphangiectasia with intestinal and pulmonary involvement in an adolescent female, who presented with many of the classic features including chylous pleural effusions, lymphopenia, hypogammaglobinemia, and a protein-losing enteropathy. She also presented with recurrent lower gastrointestinal bleeding, which is infrequently described. The patient did not improve with bowel rest and a low-fat medium-chain triglyceride diet and had little improvement with octreotide acetate therapy. However, she had a clinical response to antiplasmin therapy, trans-4-aminothylcyclohexamine carboxylic acid (tranexamic acid) in terms of serum albumin and gastrointestinal bleeding. She continues to have exacerbations of her condition, as well as persistent lymphopenia and chronic pleural effusions.

Adolescent↗
PubMed2005

Increased expression of urinary matrix metalloproteinases parallels the extent and activity of vascular anomalies.

OBJECTIVE: Matrix metalloproteinases (MMPs) and the angiogenic proteins basic fibroblast growth factor (bFGF) and vascular endothelial growth factor (VEGF) have been implicated in mechanisms of human cancer and metastasis. Assays were conducted on the urine of patients with vascular anomalies (tumors and malformations), relatively common and occasionally life-threatening disorders for which few therapies exist. We sought to determine whether these angiogenesis modulators are present in the urine and whether their expression is associated with the extent and clinical course of the vascular lesion. METHODS: A total of 217 patients with vascular anomalies and 74 age-matched control subjects participated. Urinary MMP expression was determined by substrate gel electrophoresis. Urinary bFGF and VEGF levels were measured by enzyme-linked immunosorbent assay. Each patient was assigned to 1 of 2 categories (tumor or malformation) and 1 of 9 specific groups. Extent of the vascular lesion and activity were scored by a blinded clinician. RESULTS: Urinary high molecular weight (hMW) MMPs and bFGF were significantly increased in patients with vascular tumors (53%) and vascular malformations (41%), compared with control subjects (22%). These percentages increased as a function of extent of the lesion and disease activity. hMW MMPs were increased in 4 groups: infantile hemangioma, other vascular neoplasms, lymphatic malformation and capillary-lymphaticovenous malformations, and extensive and unremitting capillary malformation and arteriovenous malformation. No significant differences among the groups were detected for low molecular weight MMPs or VEGF. CONCLUSIONS: Expression patterns of hMW MMPs and bFGF in the urine of patients with tumors and malformations are consistent with their different clinical behavior. These data represent the first evidence that MMPs are elevated in the urine of children with vascular anomalies. These data also suggest that the increased expression of urinary MMPs parallels the extent and activity of vascular anomalies in children. In addition to tumors, vascular malformations are angiogenesis dependent, suggesting that progression of a vascular malformation might be suppressed by angiogenic inhibitors, which would target bFGF and MMPs.

Adolescent↗
PubMedDate not supplied

[Chylous effusions].

This paper presents 4 patients with chylothorax, and one patient with bilateral chylothorax and chyloperitoneum. The chylous effusions were of benign etiology, developed as a complication of miliary tuberculosis (1 patient), after L-2 vertebral body fracture (1 patient), and idiopathic (2 patients). The diagnosis was confirmed by the presence of chylomicrons and high content of triglycerides in the effusion, ranged 11.9-29.1 mmol/l. Lymphangiography showed multiple abnormalities of lymphatic system, the obstruction of ductus thoracicus, dilatation and convulsion of lymphatic channels, but the site of lymphatic leak was not detected. The treatment included an extended period of pleural and peritoneal drainage with total parenteral nutrition (1 patient), pleurodesis using Corynebacterium parvum (2 patients), and surgical partial parietal pleurectomy with continuous drainage (1 patient). The treatment was successful in all patients.

Adult↗
PubMed2004

An appropriate diagnostic workup for suspected vascular birthmarks.

Birthmarks are common and commonly ignored by patients and primary care doctors. Yet they sometimes represent significant vascular anomalies that require diagnosis and treatment. We summarize when and how to work up a variety of vascular anomalies.

Arteriovenous Malformations↗